Retroperitoneal lymphatic malformations are rare benign cystic lesions of vascular origin that show lymphatic differentiation. For a broader discussion, please refer to the parental article on lymphangioma.
These malformations were formerly called lymphangiomas. This expression is out-of-date according to the 2018-classification of ISSVA 4.
Only about 1% of all lymphatic malformations occur in the retroperitoneal space. Abdominal lymphatic malformations, in total estimated in ~ 5% of cases, tend to happen more frequently in the mesentery, followed by the omentum, mesocolon, and, then retroperitoneum 1,3.
They tend to be asymptomatic, incidentally detected on imaging. Rarely, they can present as a palpable mass 3.
Lymphatic malformations are well-defined multi-locular cystic masses with typically homogeneous fluid content. Post-contrast enhancement of the cyst wall and septa may be seen 1,3. It is important to remember that they can cross fascial planes and involve multiple compartments 3.
Retroperitoneal lymphatic malformations tend to be large elongated lesions that traverse adjacent anatomic compartments.
Please refer to the article on cystic retroperitoneal lesions, for a comprehensive list of differentials.
- 1. Angela D. Levy, Vito Cantisani, Markku Miettinen. Abdominal Lymphangiomas: Imaging Features with Pathologic Correlation. (2012) American Journal of Roentgenology. 182 (6): 1485-91. doi:10.2214/ajr.182.6.1821485 - Pubmed
- 2. Dal Mo Yang, Dong Hae Jung, Hana Kim, Jee Hee Kang, Sun Ho Kim, Ji Hye Kim, Hee Young Hwang. Retroperitoneal Cystic Masses: CT, Clinical, and Pathologic Findings and Literature Review1. (2004) RadioGraphics. 24 (5): 1353-65. doi:10.1148/rg.245045017 - Pubmed
- 3. Tarek M. Hegazi, Abdulaziz M. Al-Sharydah, Karen S. Lee, Koenraad Mortele. Retroperitoneal cystic masses: magnetic resonance imaging features. (2019) Abdominal Radiology. doi:10.1007/s00261-019-02246-2 - Pubmed
- 4. ISSVA classification for vascular anomalies © (Approved at the 20th ISSVA Workshop, Melbourne, April 2014, last revision May 2018) https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf